Hmdb loader
You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2006-05-22 15:12:24 UTC
Update Date2021-09-07 16:45:34 UTC
HMDB IDHMDB0002991
Secondary Accession Numbers
  • HMDB0060150
  • HMDB02991
  • HMDB60150
Metabolite Identification
Common NameCysteamine
DescriptionCysteamine is a product of the constitutive degradation of coenzyme A, a process that occurs in all tissues, although some tissues such as brain and heart may have exceptionally high coenzyme A turnover rates. Cysteamine has only one known function, and that is as a precursor for the formation of hypotaurine, which is subsequently oxidized to taurine. The rate of cysteamine production as a result of coenzyme A breakdown is not well understood but it is clear that cysteamine levels are not as dramatically affected by dietary habits as are cysteine levels. Cysteamine is generated from hypotaurine by cysteamine dioxygenase (EC:1.13.11.19), an enzyme that was recently identified in mammals (PMID:17581819 ). Cysteamine is the simplest stable aminothiol found in the body. It is used in the treatment of disorders of cystine excretion. Cysteamine cleaves the disulfide bond with cysteine to produce molecules that can escape the metabolic defect in cystinosis and cystinuria. Cyst(e)amine may also serve as an endogenous regulator of immune system activity as well as a potential therapeutic agent for the treatment of Huntington disease. Cysteamine is also used as a radiation-protective agent that oxidizes in air to form cystamine. It can be given intravenously or orally to treat radiation sickness.
Structure
Data?1582752260
Synonyms
ValueSource
2-Amino-1-ethanethiolChEBI
2-AMINO-ethanethiolChEBI
2-AminoethanethiolChEBI
beta-AminoethanethiolChEBI
beta-AminoethylthiolChEBI
beta-MEAChEBI
beta-MercaptoethylamineChEBI
MEAChEBI
MercaptaminaChEBI
MercaptamineChEBI
MercaptaminumChEBI
ThioethanolamineChEBI
b-AminoethanethiolGenerator
Β-aminoethanethiolGenerator
b-AminoethylthiolGenerator
Β-aminoethylthiolGenerator
b-MEAGenerator
Β-meaGenerator
b-MercaptoethylamineGenerator
Β-mercaptoethylamineGenerator
(2-Mercaptoethyl)amineHMDB
2-Aminoethyl mercaptanHMDB
2-MercaptoethanamineHMDB
Aminoethyl mercaptanHMDB
BecaptanHMDB
CASHHMDB
CisteaminaHMDB
CysteamideHMDB
CysteaminHMDB
CysteaminiumHMDB
CysteinamineHMDB
DecarboxycysteineHMDB
EthanethiolamineHMDB
LambratenHMDB
LambrateneHMDB
MecramineHMDB
MercaminHMDB
MercamineHMDB
MercaptaminHMDB
MerkaminHMDB
RiaconHMDB
35S-Labeled cysteamineHMDB
CystagonHMDB
Cysteamine hydrobromideHMDB
Hydrochloride, cysteamineHMDB
Tosylate, cysteamineHMDB
Cysteamine dihydrochlorideHMDB
Cysteamine hydrochlorideHMDB
Cysteamine maleate (1:1)HMDB
Cysteamine tosylateHMDB
MercaptoethylamineHMDB
2 AminoethanethiolHMDB
Cysteamine bitartrateHMDB
Cysteamine tartrateHMDB
Cysteamine tartrate (1:1)HMDB
Hydrobromide, cysteamineHMDB
Tartrate, cysteamineHMDB
beta MercaptoethylamineHMDB
Bitartrate, cysteamineHMDB
Cysteamine, 35S labeledHMDB
Cysteamine, 35S-labeledHMDB
Dihydrochloride, cysteamineHMDB
Chemical FormulaC2H7NS
Average Molecular Weight77.149
Monoisotopic Molecular Weight77.029919919
IUPAC Name2-aminoethane-1-thiol
Traditional Namecysteamine
CAS Registry Number60-23-1
SMILES
NCCS
InChI Identifier
InChI=1S/C2H7NS/c3-1-2-4/h4H,1-3H2
InChI KeyUFULAYFCSOUIOV-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as alkylthiols. These are organic compounds containing the thiol functional group linked to an alkyl chain.
KingdomOrganic compounds
Super ClassOrganosulfur compounds
ClassThiols
Sub ClassAlkylthiols
Direct ParentAlkylthiols
Alternative Parents
Substituents
  • Alkylthiol
  • Organic nitrogen compound
  • Organopnictogen compound
  • Hydrocarbon derivative
  • Primary amine
  • Organonitrogen compound
  • Primary aliphatic amine
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological location

Source

Route of exposure

Process

Naturally occurring process

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point98 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility23.5 g/LALOGPS
logP10(0.01) g/LALOGPS
logP10(-0.42) g/LChemAxon
logS10(-0.52) g/LALOGPS
pKa (Strongest Acidic)9.42ChemAxon
pKa (Strongest Basic)10.4ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count1ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area26.02 ŲChemAxon
Rotatable Bond Count1ChemAxon
Refractivity22.39 m³·mol⁻¹ChemAxon
Polarizability8.65 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+112.60331661259
DarkChem[M-H]-105.85431661259

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
CysteamineNCCS1313.3Standard polar33892256
CysteamineNCCS686.8Standard non polar33892256
CysteamineNCCS767.8Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Cysteamine,1TMS,isomer #1C[Si](C)(C)SCCN999.4Semi standard non polar33892256
Cysteamine,1TMS,isomer #1C[Si](C)(C)SCCN1000.8Standard non polar33892256
Cysteamine,1TMS,isomer #1C[Si](C)(C)SCCN1500.5Standard polar33892256
Cysteamine,1TMS,isomer #2C[Si](C)(C)NCCS1046.5Semi standard non polar33892256
Cysteamine,1TMS,isomer #2C[Si](C)(C)NCCS887.9Standard non polar33892256
Cysteamine,1TMS,isomer #2C[Si](C)(C)NCCS1266.8Standard polar33892256
Cysteamine,2TMS,isomer #1C[Si](C)(C)NCCS[Si](C)(C)C1203.6Semi standard non polar33892256
Cysteamine,2TMS,isomer #1C[Si](C)(C)NCCS[Si](C)(C)C1217.9Standard non polar33892256
Cysteamine,2TMS,isomer #1C[Si](C)(C)NCCS[Si](C)(C)C1253.5Standard polar33892256
Cysteamine,2TMS,isomer #2C[Si](C)(C)N(CCS)[Si](C)(C)C1270.3Semi standard non polar33892256
Cysteamine,2TMS,isomer #2C[Si](C)(C)N(CCS)[Si](C)(C)C1210.9Standard non polar33892256
Cysteamine,2TMS,isomer #2C[Si](C)(C)N(CCS)[Si](C)(C)C1279.0Standard polar33892256
Cysteamine,3TMS,isomer #1C[Si](C)(C)SCCN([Si](C)(C)C)[Si](C)(C)C1467.9Semi standard non polar33892256
Cysteamine,3TMS,isomer #1C[Si](C)(C)SCCN([Si](C)(C)C)[Si](C)(C)C1465.6Standard non polar33892256
Cysteamine,3TMS,isomer #1C[Si](C)(C)SCCN([Si](C)(C)C)[Si](C)(C)C1295.4Standard polar33892256
Cysteamine,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)SCCN1206.7Semi standard non polar33892256
Cysteamine,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)SCCN1250.7Standard non polar33892256
Cysteamine,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)SCCN1643.3Standard polar33892256
Cysteamine,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)NCCS1229.9Semi standard non polar33892256
Cysteamine,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)NCCS1152.8Standard non polar33892256
Cysteamine,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)NCCS1445.3Standard polar33892256
Cysteamine,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)NCCS[Si](C)(C)C(C)(C)C1671.0Semi standard non polar33892256
Cysteamine,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)NCCS[Si](C)(C)C(C)(C)C1624.5Standard non polar33892256
Cysteamine,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)NCCS[Si](C)(C)C(C)(C)C1521.9Standard polar33892256
Cysteamine,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)N(CCS)[Si](C)(C)C(C)(C)C1670.3Semi standard non polar33892256
Cysteamine,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)N(CCS)[Si](C)(C)C(C)(C)C1619.0Standard non polar33892256
Cysteamine,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)N(CCS)[Si](C)(C)C(C)(C)C1487.2Standard polar33892256
Cysteamine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)SCCN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2090.0Semi standard non polar33892256
Cysteamine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)SCCN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2051.6Standard non polar33892256
Cysteamine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)SCCN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1687.2Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-1900000000-287334efed4c27d47e622014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-1900000000-e26f666cab18d523e4752014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-7900000000-25e27a3413a3e9bd6e862014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-MS (3 TMS)splash10-00dr-3900000000-5f3c0dc6b99382c852e22014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-EI-TOF (Non-derivatized)splash10-00di-1900000000-287334efed4c27d47e622017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-EI-TOF (Non-derivatized)splash10-00di-1900000000-e26f666cab18d523e4752017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-EI-TOF (Non-derivatized)splash10-00di-7900000000-25e27a3413a3e9bd6e862017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-MS (Non-derivatized)splash10-00dr-3900000000-5f3c0dc6b99382c852e22017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-MS (Non-derivatized)splash10-00dr-3900000000-5f3c0dc6b99382c852e22017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Cysteamine GC-EI-TOF (Non-derivatized)splash10-00di-2900000000-ba8ae281d3d77ff202c12017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Cysteamine GC-MS (Non-derivatized) - 70eV, Positivesplash10-003u-9000000000-48a76c3cb5971165dc162017-08-28Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Cysteamine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Cysteamine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-03di-9000000000-0fdf5bdcf8a4a1bf9afb2012-07-25HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Cysteamine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-03di-9000000000-f99f2f10c6728595d6ea2012-07-25HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Cysteamine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-01t9-9000000000-fa7f96e00debe1bebf702012-07-25HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Cysteamine 35V, Positive-QTOFsplash10-0udi-9000000000-f7ecadf4e8c1d670f5a72021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 10V, Positive-QTOFsplash10-004i-9000000000-9bcb6f0736d473b670412017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 20V, Positive-QTOFsplash10-01t9-9000000000-2607b3d0e4b061c850bd2017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 40V, Positive-QTOFsplash10-03di-9000000000-1455192f11f88cd089c52017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 10V, Negative-QTOFsplash10-004i-9000000000-e7f199c21a894c5cf75c2017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 20V, Negative-QTOFsplash10-004l-9000000000-148162e343b3a9f7373b2017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 40V, Negative-QTOFsplash10-001l-9000000000-205aabf68c202f080d992017-07-26Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 10V, Negative-QTOFsplash10-004i-9000000000-b167802490bdc502828d2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 20V, Negative-QTOFsplash10-00b9-9000000000-006d44449441398ce13b2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 40V, Negative-QTOFsplash10-057i-9000000000-b2c213fcf6e17049140b2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 10V, Positive-QTOFsplash10-03di-9000000000-ff9c437be9955d96c16e2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 20V, Positive-QTOFsplash10-03di-9000000000-ff9c437be9955d96c16e2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Cysteamine 40V, Positive-QTOFsplash10-03di-9000000000-947d309fccf2ffba996f2021-09-22Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 500 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Urine
Tissue Locations
  • Leukocyte
  • Pancreas
  • Skeletal Muscle
  • Spleen
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified<1 nmol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDDB00847
Phenol Explorer Compound IDNot Available
FooDB IDFDB023091
KNApSAcK IDNot Available
Chemspider ID5834
KEGG Compound IDC01678
BioCyc IDCPD-239
BiGG ID1808054
Wikipedia LinkCysteamine
METLIN ID3222
PubChem Compound6058
PDB IDNot Available
ChEBI ID17141
Food Biomarker OntologyNot Available
VMH IDCYSAM
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Dominy JE Jr, Simmons CR, Hirschberger LL, Hwang J, Coloso RM, Stipanuk MH: Discovery and characterization of a second mammalian thiol dioxygenase, cysteamine dioxygenase. J Biol Chem. 2007 Aug 31;282(35):25189-98. Epub 2007 Jun 20. [PubMed:17581819 ]
  2. Pastore A, Massoud R, Motti C, Lo Russo A, Fucci G, Cortese C, Federici G: Fully automated assay for total homocysteine, cysteine, cysteinylglycine, glutathione, cysteamine, and 2-mercaptopropionylglycine in plasma and urine. Clin Chem. 1998 Apr;44(4):825-32. [PubMed:9554495 ]
  3. Sonies BC, Almajid P, Kleta R, Bernardini I, Gahl WA: Swallowing dysfunction in 101 patients with nephropathic cystinosis: benefit of long-term cysteamine therapy. Medicine (Baltimore). 2005 May;84(3):137-46. [PubMed:15879904 ]
  4. Lochman P, Adam T, Friedecky D, Hlidkova E, Skopkova Z: High-throughput capillary electrophoretic method for determination of total aminothiols in plasma and urine. Electrophoresis. 2003 Apr;24(7-8):1200-7. [PubMed:12707912 ]
  5. Chakrabarti MC, Le N, Paik CH, De Graff WG, Carrasquillo JA: Prevention of radiolysis of monoclonal antibody during labeling. J Nucl Med. 1996 Aug;37(8):1384-8. [PubMed:8708780 ]
  6. Skov Olsen P: Role of epidermal growth factor in gastroduodenal mucosal protection. J Clin Gastroenterol. 1988;10 Suppl 1:S146-51. [PubMed:3053882 ]
  7. Gahl WA, Schneider JA, Schulman JD, Thoene JG, Reed GF: Predicted reciprocal serum creatinine at age 10 years as a measure of renal function in children with nephropathic cystinosis treated with oral cysteamine. Pediatr Nephrol. 1990 Mar;4(2):129-35. [PubMed:2397178 ]
  8. Smolin LA, Clark KF, Thoene JG, Gahl WA, Schneider JA: A comparison of the effectiveness of cysteamine and phosphocysteamine in elevating plasma cysteamine concentration and decreasing leukocyte free cystine in nephropathic cystinosis. Pediatr Res. 1988 Jun;23(6):616-20. [PubMed:3393396 ]
  9. Levtchenko EN, van Dael CM, de Graaf-Hess AC, Wilmer MJ, van den Heuvel LP, Monnens LA, Blom HJ: Strict cysteamine dose regimen is required to prevent nocturnal cystine accumulation in cystinosis. Pediatr Nephrol. 2006 Jan;21(1):110-3. Epub 2005 Oct 27. [PubMed:16252107 ]
  10. Liu YC, Wang CM, Hsiung KP: Comparison of different protein immobilization methods on quartz crystal microbalance surface in flow injection immunoassay. Anal Biochem. 2001 Dec 15;299(2):130-5. [PubMed:11730334 ]
  11. Yudkoff M, Foreman JW, Segal S: Effects of cysteamine therapy in nephropathic cystinosis. N Engl J Med. 1981 Jan 15;304(3):141-5. [PubMed:7442733 ]
  12. Gahl WA, Charnas L, Markello TC, Bernardini I, Ishak KG, Dalakas MC: Parenchymal organ cystine depletion with long-term cysteamine therapy. Biochem Med Metab Biol. 1992 Dec;48(3):275-85. [PubMed:1476793 ]
  13. Geelen JM, Monnens LA, Levtchenko EN: Follow-up and treatment of adults with cystinosis in the Netherlands. Nephrol Dial Transplant. 2002 Oct;17(10):1766-70. [PubMed:12270982 ]
  14. Stachowicz M, Lehmann B, Tibi A, Prognon P, Daurat V, Pradeau D: Determination of total cysteamine in human serum by a high-performance liquid chromatography with fluorescence detection. J Pharm Biomed Anal. 1998 Aug;17(4-5):767-73. [PubMed:9682161 ]
  15. Orloff S, Butler JD, Towne D, Mukherjee AB, Schulman JD: Pantetheinase activity and cysteamine content in cystinotic and normal fibroblasts and leukocytes. Pediatr Res. 1981 Jul;15(7):1063-7. [PubMed:7254953 ]
  16. de Graaf-Hess A, Trijbels F, Blom H: New method for determining cystine in leukocytes and fibroblasts. Clin Chem. 1999 Dec;45(12):2224-8. [PubMed:10585356 ]
  17. Chmiel J, Kopczynski Z, Rybczynska M: The influence of the known radioprotective compounds on the metabolism of red blood cells. I. Effect of cysteamine on the cellular level of the intermediates and coenzymes. Pol J Pharmacol Pharm. 1976;28(2):113-21. [PubMed:934944 ]
  18. Schneider JA, Clark KF, Greene AA, Reisch JS, Markello TC, Gahl WA, Thoene JG, Noonan PK, Berry KA: Recent advances in the treatment of cystinosis. J Inherit Metab Dis. 1995;18(4):387-97. [PubMed:7494398 ]
  19. Thiele I, Swainston N, Fleming RM, Hoppe A, Sahoo S, Aurich MK, Haraldsdottir H, Mo ML, Rolfsson O, Stobbe MD, Thorleifsson SG, Agren R, Bolling C, Bordel S, Chavali AK, Dobson P, Dunn WB, Endler L, Hala D, Hucka M, Hull D, Jameson D, Jamshidi N, Jonsson JJ, Juty N, Keating S, Nookaew I, Le Novere N, Malys N, Mazein A, Papin JA, Price ND, Selkov E Sr, Sigurdsson MI, Simeonidis E, Sonnenschein N, Smallbone K, Sorokin A, van Beek JH, Weichart D, Goryanin I, Nielsen J, Westerhoff HV, Kell DB, Mendes P, Palsson BO: A community-driven global reconstruction of human metabolism. Nat Biotechnol. 2013 May;31(5):419-25. doi: 10.1038/nbt.2488. Epub 2013 Mar 3. [PubMed:23455439 ]

Enzymes

General function:
Involved in hormone activity
Specific function:
Somatostatin inhibits the release of somatotropin
Gene Name:
SST
Uniprot ID:
P61278
Molecular weight:
12735.4
General function:
Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides
Specific function:
Amidohydrolase that hydrolyzes specifically one of the carboamide linkages in D-pantetheine thus recycling pantothenic acid (vitamin B5) and releasing cysteamine.
Gene Name:
VNN1
Uniprot ID:
O95497
Molecular weight:
57011.16
Reactions
Pantetheine + Water → Pantothenic acid + Cysteaminedetails
General function:
Involved in cysteamine dioxygenase activity
Specific function:
Not Available
Gene Name:
ADO
Uniprot ID:
Q96SZ5
Molecular weight:
29750.535
Reactions
Cysteamine + Oxygen → Hypotaurinedetails
General function:
Not Available
Specific function:
Amidohydrolase that hydrolyzes specifically one of the carboamide linkages in D-pantetheine thus recycling pantothenic acid (vitamin B5) and releasing cysteamine. Involved in the thymus homing of bone marrow cells. May regulate beta-2 integrin-mediated cell adhesion, migration and motility of neutrophil.
Gene Name:
VNN2
Uniprot ID:
O95498
Molecular weight:
33256.93
Reactions
Pantetheine + Water → Pantothenic acid + Cysteaminedetails
General function:
Not Available
Specific function:
Amidohydrolase that hydrolyzes specifically one of the carboamide linkages in D-pantetheine thus recycling pantothenic acid (vitamin B5) and releasing cysteamine.
Gene Name:
VNN3
Uniprot ID:
Q9NY84
Molecular weight:
Not Available
Reactions
Pantetheine + Water → Pantothenic acid + Cysteaminedetails